Henoch-Schönlein Purpura in children: Not only kidney but also lung

Giada Maria Di Pietro, Massimo Luca Castellazzi, Antonio Mastrangelo, Giovanni Montini, Paola Marchisio, Claudia Tagliabue

Research output: Contribution to journalReview articlepeer-review


Background: Henoch-Schönlein Purpura (HSP) is the most common vasculitis of childhood and affects the small blood vessels. Pulmonary involvement is a rare complication of HSP and diffuse alveolar hemorrhage (DAH) is the most frequent clinical presentation. Little is known about the real incidence of lung involvement during HSP in the pediatric age and about its diagnosis, management and outcome. Methods: In order to discuss the main clinical findings and the diagnosis and management of lung involvement in children with HSP, we performed a review of the literature of the last 40 years. Results: We identified 23 pediatric cases of HSP with lung involvement. DAH was the most frequent clinical presentation of the disease. Although it can be identified by chest x-ray (CXR), bronchoalveolar lavage (BAL) is the gold standard for diagnosis. Pulse methylprednisolone is the first-line of therapy in children with DAH. An immunosuppressive regimen consisting of cyclophosphamide or azathioprine plus corticosteroids is required when respiratory failure occurs. Four of the twenty-three patients died, while 18 children had a resolution of the pulmonary involvement. Conclusions: DAH is a life-threatening complication of HSP. Prompt diagnosis and adequate treatment are essential in order to achieve the best outcome.

Original languageEnglish
Article number75
JournalPediatric Rheumatology
Issue number1
Publication statusPublished - Nov 21 2019


  • Children
  • Diffuse alveolar hemorrhage
  • Henoch-Schönlein Purpura
  • IgA Vasculitis
  • Pulmonary involvement

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Rheumatology
  • Immunology and Allergy


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